{"orpha":99791,"name":"Dentin dysplasia type II","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["DD-II","DTDP2"],"definition":"Dentin dysplasia type II (DD-II) is a rare mild form of dentin dysplasia (DD) characterized by normal tooth roots but abnormal primary dentition.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99791/","orphanet_url":"https://www.orpha.net/en/disease/detail/99791","names":{"fr":{"name":"Dysplasie de la dentine type II","synonyms":["DD-II","DTDP2","Dysplasie dentinaire type II"],"definition":"Forme rare et bénigne de dysplasie dentinaire (DD) caractérisée par des racines dentaires normales, mais des dents de lait anormales."},"de":{"name":"Dentindysplasie Typ 2","synonyms":["DTDP2"],"definition":"Die Dentindysplasie Typ II (DD-II) ist eine seltene milde Form der Dentindysplasie (DD, siehe diesen Begriff), gekennzeichnet durch normale Zahnwurzeln, aber ein abnormes Milchgebiss."},"es":{"name":"Displasia de la dentina tipo 2","definition":"La displasia de la dentina tipo II (DD-II) es una forma leve, poco frecuente, de la displasia de la dentina (DD; ver este término) caracterizada por dientes con raíces normales pero con una dentición primaria anómala."},"it":{"name":"Displasia della dentina, tipo II","definition":"La displasia della dentina tipo II (DD-II) è una forma rara e lieve di displasia della dentina (DD; si veda questo termine), caratterizzata da denti con radici normali e difetti della dentizione primaria."},"nl":{"name":"Dentinedysplasie type II","synonyms":["DD-II","DTDP2"],"definition":"Dentinedysplasie type II (DD-II) is een zeldzame milde vorm van dentinedysplasie (DD, zie deze term), gekenmerkt door normale tandwortels maar abnormale melktanden."},"pt":{"name":"Displasia da dentina, tipo II"},"pl":{"name":"Dysplazja zębiny typu II","synonyms":["DD-II","DTDP2"]},"cs":{"name":"Dentinová dysplázie, typ II","synonyms":["DD-II","DTDP2"]},"uk":{"name":"Дисплазія дентину, тип II","synonyms":["DD-II","DTDP2"],"definition":"Dentin dysplasia type II (DD-II) is a rare mild form of dentin dysplasia (DD, see this term) characterized by normal tooth roots but abnormal primary dentition."},"zh":{"name":"牙本质发育异常2型","synonyms":["DTDP2"],"definition":"牙本质发育不良II型(DD-II)是一种罕见的轻度牙本质发育不良(DD，见该词条)，其特征是牙根正常，但乳牙牙列异常。"},"tr":{"name":"Dental displazi tip II","synonyms":["DD-II","DTDP2"]}},"xrefs":[{"source":"OMIM","code":"125420","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"K00.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA30.7","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"227906909"},{"source":"MONDO","code":"0007437","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0399380","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"1806","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98026,"name":"Rare odontologic disease"},"parents":[{"orpha":1653,"name":"Dentin dysplasia","classifications":[156,197]}],"children":[],"genes":[{"symbol":"DSPP","name":"dentin sialophosphoprotein","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":null,"hgnc":"3054","ensembl":"ENSG00000152591","omim":"125485","loci":["4q22.1"]}],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":[],"prevalence":[{"type":"Cases/families","qualification":"Family(ies)","class":null,"value":19.0,"area":"Worldwide","status":"Validated","source":"10397672[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}